Paper - Horseshoe Kidney

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Eisendrath DN Phifer FM and Culver HB. Horseshoe Kidney (1925) Ann Surg. 82(5): 735-64. PubMed 17865363

Horseshoe Kidney

By Daniel N. Eisendrath, M.D., Frank M. Phifer, M.D. Harry B. Culver, M.D.

of Chicago, Ill.

From the Cook County and Michael Reese Hospitals.

Definition

AS stated in a previous article we believe that the term “fused kidney” should be discarded. In its place we should employ the

following terms to designate the respective conditions: I. Crossed F.ctopia.—To be used for those cases in which both kidneys

fiG. I.—Horscshoo kidney with symmetric halves. (Drawing made from specimen in Rush Medical College Museum.)

are found on the same side of the body. They may be fused into one mass or be separated. The lower of the two kidneys corresponds to the one which in the embryo should have been found on the opposite side of the body.

2. Double Kidney.——To be used for those cases in which there is a reduplication either complete or incomplete of the ureter and a corresponding reduplication of the renal pelvis on one or both sides of the body. The parenchyma around the respective pelves of each half of the kidney may fuse, or the two halves may be more or less separated.

3. Horseshoe Kidney.—The two kidneys of opposite sides of the body are connected across the spine by an isthmus which may consist only of

fiG. 2a.—Horseshoe]kidneys with asymmetric halves. One-ha.lf_'at higher level. (Rush Medical College Museum.)

fibrous tissue or of parenchyma. The isthmus varies greatly in width and as to whether it connects the upper or lower poles.

4. Cake or L. Kidney.—These are simply sub-varieties of the horseshoe kidney. If the isthmus which extends across the spine is so wide that it connects the two kidneys along their entire mesial borders, we speak of a cake kidney. (fig. 7.) If one-half of the horseshoe kidney is elongated so that the other half only is united to its lowermost portion, we speak of an L. kidney.

(B of fig. 2.) 1. Frequency of Horseshoe Kid12ey.—Botez°" collected the statistics of

fiG. 2b.—-Horseshoekidneys with asymmetric halves. The two halves form an L-shaped mass. (Garre and Ehrhardt case.)

51,504 autopsies published by various authors up to, 1912. Horseshoe kidney was found in 72 of these, or I to 715 autopsies. Carlier and Gerard,3 in 1913, added some later observations to those of Botez, finding that this anomaly occurred eighty times in 69,98'9 autopsies or I to 862. Since I913,

fiG. 4.—Horseshoe kidney with superior isthmus. (Byron Robinson case.)

738 HORSESHOE KIDNEY

the observation of Motzfeld “ can be added, making a total of 73,489 autopsies in which horseshoe kidney was found in 92, or approximately I in 710 bodies.

2. Relation of the Two Halves-—Horseshoe kidneys may be divided as follows: i l l

I. Symmetric.—Both halves approximately equal‘ in size and at the same level. (fig. I.)

2. Asymmetric. —Inequality in size and level of the two halvesf (-fig. 2.) One side may be hypoplastic and the other the size of a normal kidney, or one side may be of normal size and the other so elongated as to form together a V or L-shaped mass. ' h (fig. 2.) ‘ 1

As a rule the two‘ halves are situated an equal distance from the spine, but it is well to remember in our radiographic study of suspected it cases that one or both halves may be as far away i‘ * from the spine as is the normal kidney, or on the other hand, that one-half may be quite close to the spine andthe other not. (fig. I4.) It is" not 1111 Colnrnon t0 find 3 h,V])0" fiG. )5.-—'-Isthmu_s_corC111p1c:sed of botlli cortel.-3x and ‘medulla. Tlhe isth° ' t . . plasla of one-half and a musjome eupper Po es ( aetzner Case) ‘

compensatory increase in size of the other half. As a rule the lower poles converge, as is true in the embryo (Broman) and: hence the renal shadows and pyelograms or calculous shadows fig. I5) are often directedobliquely inwards. The upper poles _in some cases are very far apart and the angle which the halves form with the spine wider than when the upper poles are a normal distance apart.

3. The Isthmus, etc.—(a) Inferior and superior. Byron Robinson 5 found

the isthmus joining the lower poles in 88 per cent. of his observatioiis,

Beyer *3 found such an inferior isthmus in 93 per cent. and Gerard in 91 per cent._. so that one can say that it is so located in about 90 percent.“ of the cases. The superior polar isthmus occurs in the remaining 10 per cent. (See fig. 3.) if 4' if s (b) Width and Character ‘of Isthmus.-This was fibrous in (fig. 4) seven cases. Robinson (loc. cit.) estimates that this condition exists in I 5 per cent. of all cases, but this appears too high a percentage. In.the majority of cases the isthmus is composed of parenchyma, so that there is no demarcation between the two halves. (fig. 5.) The isthmus in a vertical direction measures from 2 to 3 cm. in the majority of cases.

fiG. 6a.—Specimen in_ Rush Medical College Museum, illustrating variation in width of isthmus. Compare with figs. 3, 6b and 7 to understand how the cake kidney originates.

fiG. 6b.—-Specimen in.Rush Medical College Museum, illustrating variation in width of isthmus. Cornpare with figs. 3, 6a and 7 to understand how the cake kidney originates.

(c) Transition to Cake K'idney.—The isthmus may unite a variable proportion of the two halves, as a rule only the poles, but it may fuse together more than the 2 to 3 cm. just mentioned so that all degrees (fig. 6, a and b) are found up to that of complete fusion to which the name cake kidney (fig. 7) has been given. Here there is a solid mass of renal tissue without any mesial demarcation.

(d) Relation of Isthnms to A0rta.—In only two cases, ‘via, those of Nixon 7 and Kelly3 was the isthmus behind the aorta.

4. Renal Pelvies.-In the majority of cases there is a single pelvis on each side. (fig. I.) Reduplication of the ureters and of the pelves on one or both sides is not rare. (fig. 8.)

The pelvis is usually on the anterior (ventral) aspect of -the kid n e 3' (fig. I) at the level of the normal hilus. and resembles that of the normal organ in respect to being a single cavity with its calyces, located either partly external to the hilus or not extending beyond it; i.e., intrar e n a 1. In horseshoe kidney a true pelvis of this kind is often al)sent,'the calyces being all extrarenal and ending independently in the ureter. (fig. 9.)

5. Ureters.—As a rule the ureters pass across the front of the isthmus and this accounts for the frequency with which calculi, hydronephrosis, etc., occur. Robinson found that the ureters passed behind the isthmus (fig. 10) in 9 per cent. of his specimens, but this figure would seem too high inasmuch as only two reports, 1/z'z., those of Landouzy° and Durham 1° have been published of ureters behind the isthmus. The latter according to Robinson and other observers, at times has an independent ureter. In Karl _Ioseph’s case this isthmian ureter ended independently in the bladder. Perruchet 11

tum

5'l_F1G. 7. Typical cake kidney. (Papin.)

describes a case in which one ureter passed behind the other in front of the isthmus. As a rule calyces are only present in the upper two-thirds of each half, but an extrarenal calyx or an independent ureter may drain the isthmus (fig. 9) and be opened during the operation of division of the isthmus or of heminephrectomy. The ureters usually end in the bladder at the normal location, but it must be remembered clinically that one ureter may end ectopically (fig. 11) as is so often the case in double kid-neys. A

6. Location of H orscslzoc K1'dn.cy.——Tliis is usually lower, just above the aortic bifurcation (fig. I), but it may be anywhere from the normal level of

fiG. 8.—Horseshoe kidney with two ureters'_and two’pelves for each half. (Byron Robinson case.)

the kidneys to the true pelvis. (fig. 12.) Such‘ a pelvic ectopia is not uncommon.“ Only seven cases are reported in which the isthmus was at the normal level ofthe lower poles. In Rathbun’s 1‘-’ case one-half of the.horseshoe kidney was in the true pelvis. The majority of horseshoe kidneys which lie in thetrue pelvis-are of the cake (fig. 7) variety, z'.'e., have completely fused halves. The isthmus is usually at the level of the fourth to fifth lumbar vertebrae and may not be in the median line. There is very little mobility as a rule in a horseshoe kidney, but a few cases have been reported in which marked mobility existed. The fixation of "a horseshoe kidney is in great measure due to the fact that it has multiple blood-vessels supplying it; all from immediately adjacent trunks. There is but little perinephric fat, hence this does not play a role in fixation of the horseshoe kidney.

Fig. 9. — Horseshoe kidney with eiitrare_nal calyce endng directly in left lielf, io ureter. (Rush Medical College Museum.)

Fig. 10. —Horseshoe kidneys with symmetric hglves in which urets crossed posterior aspect of isthmus. (Rush Medical College Museum.)

7. Blood Supply.—It is important from the operative standpoint to remember that multiple arteries and veins for each half and often for the isthmus as well, are found in eighty per cent. In a study of I 39 cases, including I0 of his own, Papin” found the following:

(a) A single artery for both halves in only one case. (Bruncher..)

(b) One artery for each half in 25 cases. (A of fig. I 3.)

(c) One artery for each half and one for the isthmus. (B of fig. 13.) This is almost the normal condition. There were 40 cases in this group.

(d) Two arteries for each half and one for the isthmus. (C of fig. 13.) The one for the isthmus is an aortic branch. There were 26 cases in this group.

(e) Two arteries for each half and one or two for the isthmus. The former are given off by either the aorta or the common iliacs. The latte r (isthmic branches) arise from the iliacs. (C

of fig. .13.) Twenty fiG. II.—Horseshoe kidney with relatively wide isthmus. One ureter ends just below external meatus. (Female.) (Massari C3533 belonged to this case.) group.

In the remaining groups there were from six to eight arteries for the two halves. The important deductions are that‘ one must have an adequate exposure of the operative field because (a) of the multiplicity of the vessels. both arteries and veins,which supply both halves and the isthmus, and (1)) because they may arise from the aorta or end in the vena cava, respectively, or similarly from the iliacs.

CLINICAL IMPORTANCE OF HORSESHOE KIDNEY

I. Factors Favoring Pathologic Conditions.— (a) Course of ureter across isthmus. This is perhaps the most important, because of the sharp bend which must be made by the ureter across the more or less thick and hard isthmus. (fig. I.) Infections of the kidney involving the ureter secondarily are more apt to cause obstruction through fixation and kinking than in the caseof the normal ureter.

(b) The abnormal’ location of the pelvis on the ventral aspect of the kidney and the fact that the ureteral insertion is often at a higher point than the bottom of the pelvis and the f r e q u e nt absence of a pelvis proper (fig. 9), all favor stagnation of urine and subsequent infection. (figs. 17 to 20.)

(c) The frequent occurrence 'of congenital strictures of the ureter in horseshoe kidneys. I

(d) The presence of many accessory vessels and the possibility of ureteral obstruction by them.

2. Published Clinical Cases. Botez (loc. cit.) collected all clinical reports up to 1912 and included several unpub lished Ones (Marion) in fiG. I2.—Hydronephro_sis of lefltl half of pelvic ectopic cake article. Of a total of fifty “‘d“°Y- ‘ °““”-’

of Botez’s cases, only 39 are of value from the operative standpoint. Since I912, we have found reports of ninety additional clinical cases and with our own, reported in this article, we have a total of I 32 up to July, I925. (See

Tables I to VII inclusive.)

We will report our three cases before taking up the subject any further.

CASE I.—Pyelotomy for renal calculus in one-half of a horseshoe kidney. Presence of this anomaly diagnosed before operation.

Male, aged thirty-two, complained of pain in right lumbar region radiating to right upper quadrant of abdomen, of two days’ duration. In addition to tenderness over the right iliocostal space, there were other evidences of acute renal infection. Radiography (Dr. Cora M. Matthews) revealed an oval vertical shadow (A of fig. 14) lying over the transverse process of. the second lumbar vertebra; i.e., closer to the spine than shadows of renal or ureteral calculi usually do. The right opaque catheter curved slightly out 745 746

TABLE I

Division of Isthmus Alone (Symphysiotomy) or Combined with other Operations

Author and reference

E. Papin: Assoc. franc. d’Urologie, 22nd Congress, Paris, Oct.

22. 1922, P- 557

Sex and age

Female years

32

Chief clinical data 0P€1'3ti0n

-<- 4- u‘ 4-.——:

4-4. I - n n u I —'

Ureter in front of the 2 cm. isthmus on both sides. Division of isthmus was easy. Extraperitoneal approach

Gradually increasing abdominal and lumbar pains. Could feel isthmus and confirmed diagnosis by pyelography. Both pelves lower, with calyces directed towards midline

Remarks

-4. — -4: :1. -1 -R

Complete relief of pain. Diagnosis of horseshoe kidney made by pyelography.

A Martinovv: Zent. f. Chir., 9, 314,

(Feb._) 1910

Rovsing: Zeit. f. Urol., 5, 586, I911

—-.__. a 1

Malinowsky: Jour. d'Uro1ogie, I, 869, (Dec.) 1912

Mintz: Chirourg. Archiv. Veliam, 29, I047, I923. Quoted by Papin: Arch mal. des Reins, 2, 24, Feb. I, 1925

Female years

49

Male 23 years

Female -years

Female years

28

33

u 1 x «u 4...

Recurrent attacks of pain above» level of umbilicus where could palpate tender mass » '

Transperitoneal division of isthmus

— 4—_ 1 4- 1—

Crushed isthmus by transperitoneal route

Recurrent attacks of girdlelike pain at level of umbilicus. Disappeared when in recumbent position. Could feel mass running obliquely across abdomen

Persistent abdominal pain, Crushed isthmus accompanied by hyperacidi ity. Could palpate isthmus

Recurrent attacks of nonlocalizable abdominal pain. Felt mass size child ’s head at level of umbilicus

Transperitoneal division of isthmus Complete relief of pain. Diagnosis of horseshoe kidney made by palpation alone.

—’ 1

Complete relief of pain. Diagnosis made by palpation alone.

«—_

Complete relief of pain, but not of hyperacidity. Diagnosis made by palpation alone.

1

Complete relief of pain. Diagnosis of horseshoe kidney not made before operation.

Brongersma: Zeit. f. Urol., 8, 477, Female, age Recurrent pain, (bilateral) Transperitoneal division of Complete relief of pain1914 not given resembling ureteral colics isthmus Diagnosis not made before operation.

._. . I dem.. Male, age not Pain at level of umbilicus T ransperitoneal division of Complete relief of pain. given especially upon leaning for— isthmus Diagnosis of horseshoe kidwards. Haematuria once ney made at previous abafter lifting heavy weight dominal operation.

n:

iEggers: Zeit. f. Urol. Chir., 9, 427, Male 18 years History of left-sided renal Extraperitoneal division of Recovery. Diagnosis made

1922 calculus, confirmed by radio- isthmus with fixation of left during operation. ., graphy. Pyelography after half after pyelotomy for mul operation revealed both tiple calculi

pelves _close to spine

1

Kroiss: Verh. d. Deut. Gesell. f. Female 29 Recurrent severe pain at Transperitoneal division of Complete relief of pain. Urologie, 1922 years level of umbilicus. Palpable isthmus J Possibility of horseshoe kidmass below this level. Pye- ney considered before operlography reveals both pelves tion.

much lower than normal ' I

Kidd: Proc. Royal Soc. Med., Female 32 Recurrent attacks of severe Transperitoneal division of Complete relief of pain. London, 15, 52, 1922 years pain over sacral region. Very isthmus Diagnosis made during primobile kidney to be felt in mary operation for supposed right iliac fossa mobile kidney.

' Van Houtem: Zeit. f. Urol. Chir., I Female 37 Recurrent attacks of colicky '1‘ ransperitoneal division of Gradual disappearance of 8, 165, 1922 years pain in back and over abdo- isthmus pain. Diagnosis made by men, accompanied by haem- palpation under anaesthesia l aturia. Pyelography revealed and confirmed by pye1ogone pelvis close to spine raphy before operation.

‘ De Groot: Zeit. f. Urol. Chir., Male 16 years Recurrent severe abdominal Transperitoneal division of Complete relief of pain. 8, 170, I922 pain " which disappeared isthmus Diagnosis made during exwhen in recumbent position. ploratory laparotomy. Could feel mass above and . . to left of umbilicus

TABLE II

H eminephrectomy Alone

J Case No

Author and reference

Indication for operation and remarks

Barth (Israel) :

Arch. Klin. Chir., 74, 3689 I904’ '

Tuberculous hydronephrosis of right half.

2 Clairmont: Arch. Klin. Chir., 79, 667, Hydronephrosis in two-year-old child. 1906 3 Hoffman: Wien. Klin. Woch., I22, 355, Tuberculosis. Recovered. 1913 4 Marjasches (see Kobylinski) Same as No. 3. Died p.o. 5 Koenig: Deut. Zeit. Chir., 40,92, I895 Sarcoma in child. 6 Gibbon: Rev. de Chir., 1265, 1909 Same. 7 Debuchy (see Koby1inski): Folia Urol., Carcinoma. 6, 160, 1911 8 _ Rumpel: Zent. Chir., 29, 1091, 1902 Calculous pyonephrosis. 9 Lotheissen: Arch. Klin. Chir., 52, 768, Hydronephrosis secondary to ureteral stric1896 ture. Died p.o. 1o Paschkis: Wien, Med. W., 60, 2417, Calculous pyonephrosis. Oct., 1910 11 Kiimmell: Case 2. (Flockemann) Zeit. Hydronephrosis. Recovered. Urol. Chir., 4, 204, 1918 I2 Kiimmell: Case 4. (Flockemann), Calculouslhydronephrosis. Recovered. ' I Idem.

I 3 Idem: Case 5. Idem. Chronic nephritis. Recovered.

14 Faykiss: Wien. Med. W., 60, 1479, 1914 Tuberculosis. Recovered.

15 Hi1debrandt:Zeit. f. Urol., 14, 465, 1920 Sarcoma.

16 Albarranz Ann. Mal. Gonitourin, 25, Hydronephrosis. Recovered.

8o1,19o7

17 Legueu: Necker Clinics, 1922 Tuberculosis.

I 8 Idem. Echinococcus.

19 Renton: Brit. Med. Jour., 1,601, May Calculus with atrophic pyelonephritis.

20,1920 . 20 Leedham-Green: Brit. Med. _]our., 2, Pyonephrosis.‘ 1583, Dec. 20, 1923 21 Idem. Same. 22 Bryan: Virg. Med. Month., 48, 75, Hydronephrosis. Pyeloigram (post-operaMay, 1921 tive) showed median pelvis.

TABLE II—Contz'nued

Heminephrectomy Alone

0 . 8% Author and reference Indication for operation and remarks 23 Rawlingz Brit. Jour. Surg., 9, 162, I921 Bilateral nephrolithiasis. Heminephrectomy for calculous pyonephrosis. 24 Thompson: ANNALS OF SURGERY, 54, Pyonephrosis. 355, Sept., I911 25 Harris (see Thompson) Tuberculosis. 26 Rehling: Int. Jour. Surg., 32_, 239'," I919 Hydronephrosis. Recovered. 27 Magnus: Zent. f. Chir. Tuberculosis. Died seven weeks after operation. 28 Jeck: Int. Jour. Surg., 32, 639, I919 Pyonephrosis. 29 Judd, Braasch & Scholl: J.A.M.A., 79, Ureteral calculus complicated by hydroneI 189, Oct. 7, I922 phrosis. 30 Idem. Infected hydronephrosis. 31 Idem. Multiple calculi. 32 Idem. Hydronephrosis. 33 Judd, Braasch & Scholl, Idem. Infected hydronephrosis. 34 Idem. Infected hydronephrosis. 35 Idem. Infected hydronephrosis. 36 Oraison: Gaz. Hebdom., 40, 32, Feb. Tuberculosis. 9. 1919 37 Nash: Lancet, 174, I151, I908 Hydronephrosis in 16 months child. _38 Bugbee & Losee: Surg. Gyn. & Obst., Tuberculosis. 28, 97, Feb., 1919 39 Rathbun: Jour. Urol., 12, 612, Dec., Hydronephrosis. Died 17 days p.o. Diagno1924 ' sis made before operation by pyelography. 40 Idem. Calculous pyonephrosis. 41 Hess: Jour. Urol., I2, 267, I924 Uretal calculus complicated bypyonephrosis. 42 Loelfler (Kroiss): Zeit. Urol. Chir., I6, Infected hydronephrosis due to ureteral 181, 1924 kink. 43 Idem. Tuberculosis. 44 W. Carl: Zent. f. Chir., 50, 506, Mar. Multiple calculi complicated by pyone3I, 1923 phrosis. . 45 G. Magnus: Zent. Chir., 54, 76, Jan. Tuberculosis. Died7w‘eeks p.o.

24» 1925

TABLE II—Continued Heminephrectomy Alone

Case No.

Author and reference

1_j_ 4—

Indication for operation and remarks

Leekahr: Ky. Med. Jour., 21, 679, Dec., 1923

— ——. 4——: —1-————

Marson: Brit. Med. Jour., 1, 237, Feb.

Infected hydronephrosis.

47 Infected hydronephrosis complicating cal1o, 1923' C1111 (renal). Recovery. 48 Baltscheffsky: finska. Lack. Handl., Tuberculosis. Recovery. 64,377.I922 I ' 49 Israel: Fol. Urol., 1, 617, 1908 Hydronephrosis. Diagnosis before opera' tion by palpation. 50 Idem, Tuberculosis. fistula persisted. 51 Zondek: Deut. Med. Woch., 46, 897, Calculous pyonephrosis. Aug. 5, 1920 ~ 52 Bockenheimer: Berl. Kl. Woch., 48, Hydronephrosis in boy of six, due to ureteral 641, Sept. 4, 1911 kink. 53 Simon: Beitr. Klin. Chir., 26, 148, I900 Sarcoma. Died two days p.o. of anuria. 54 Denk: Arch. Klin. Chir., I 16, 245, 1921 Shadows of multiple calculi close to spine at level of 3rd to 4th lumbar vertebrae. Found calculous pyonephrosis of one-half of horseshoe kidney and resected. Isthmus at upper poles. I 55 Karewski: Deut. Med.‘Woch., 47, 989 Infected hydronephrosis of one-half of horseshoe kidney with superior isthmus. 56 Key: Nor. Med. Ark., 47, I, 1921 Hypernephroma. 57 Gibbon: Rev. de Chir., 1265, 1909 Sarcoma. Recovered. 58 Desmarest: J. de Chir., 5, 742, 1910' Calculous hydronephrosis. Recovery. 59 Enderlen: Presse Med., 357, I910 Hydronephrosis. Recovery. 60 Marion: Unpublished but cited by Hydronephrosis. Recovery. Botez (lac. cit.) 61 Michon: Assoc. Franc. d’Urol., 15t_h Hydronephrosis. Secondary. Session, 1911 62 Oehlecker: Zeit. Urol. Chir., I0, 66, Hydronephrosis. 1922 63 Rovsing: Zeit. f. Urol., 5, 586, 1911 Pyonephrosis. Suspected horseshoe kidney from palpatory findings. 64 Gayet: Jour. d’Urol. Tuberculosis. Made diagnosis before opera tion by proximity of lower poles "(palpation).

TABLE III Pyelotomy or Nephrotomy

Q) . 53:2 Author and reference Technic and remarks

1 V. Frisch: Proc. German Urol., Con- Pyelotomy for calculus anuria. Calculus

gress, 191 I passed spontaneously later. Recovery.

2 Steiner, Idem. Nephrotomy for two large calculi. Recovery. Suspected horseshoe kidney from palpatory findings.

3 Eisendrath: Surg. Gyn. & Obst., 15, 467, Hydronephrosis. Nephrotomy.

Oct., 1912 4 Israel: Fol. Urol., 1, 617, 1908 Diagnosis by palpation before operation. Bilateral pyelotomy for calculi.

5 Zuckerkandl (Paschkis): Wien. Med. Pyelotomy for calculus.

Woch., 59, 2605, Oct. 30, 1909 6 r Perineau (Marion): Ann. Mal. Gen. Pyelotomy. Recovery. Urin., 28, 427, 1910 7 Roth (Casperz) Berl. Klin. Woch., 48, Nephrotomy for calculus. 66, Jan. 9, 1911 8 Adrian: Folia Urol., 8, 189, 1913 Pyelotomy for calculus. 9 Blesh: Jour. Okla. Med. Ass’n., I4, 239, Ureterotomy for calculus. Ureter behind Sept. 21, 1921 isthmus. I0 Krotoszyner: ANNALS OF SURGERY, 65, Nephrotomy for calculus in case of bilateral 565, 1917 renal calculus. Died on 3rd day after operation of uremia. 11 Renton: Brit. Med. ]our., 1, 601, May Nephrotomy for calculus. 20,1920 12 Taylor (Deaver): Am. J. Med. Sci., 161, Transperitoneal route. Pyelotomy for large 238, 1921 r calculi. I3 Folsom: Texas St. Med. ]our., 16, 201, Pyelotomy for multiple small calculi. Sept., 1920 14 Leedham-Green: Brit. Med. Jour., 2, Pyelotomy for ca1culus.. 1583, Dec. 20, 1913 15 Idem. Pyelotomy for calculus. 16 Newman, Lancet, 2, 236, Aug. 18, Diagnosis by palpation before operation. 1917 Nephrotomy for calculus. I7 Judd, Braasch and Schollz J.A.M.A., Nephrotomy for calculus. 791, 189, Oct. 7, 1922 18 Idem. Pyelotomy for calculus. 19 I dem? (Case 13) Diagnosis made before operation from ~ presence of shadows close to midline. Bilateral pyelotomy for calculi. 20 Pyelotomy for calculus.

Idem. (CaLsefi14)

TABLE III——Contz'nued

Pyelotomy or Nephrotomy

Case | No.

Author and reference

Technic and remarks

Idem. (Case 15)

{.1 Diagnosis made before operation because

of proximity of shadows and of one pyelogram to spine and anterior rotation of calyces. Bilateral pyelotomy for calculi.

Judd, Braasch and Scholl: Casc 16, Idem.

Kinard: J.A.M.A., 81, 2077, Dec. 22, 1923

4 Eisendrath, Culver and Phifer (Present article), Case 1 ”'

Pyelotomy for calculus.

4 ;

Pyelotomy for calculus in one-half. Shadows present in opposite kidney but operation.

—n —. -:1

Pyelotomy for multiple calculi. Diagnosis before operation from proximity of shadows to spine and pyelography.

J

Eisendrath, Culver and Phifer: (Pressent article), Case 2.

Schuchardt (See Paschkis): Wien. Med. Woch., 60,2417, Oct. 8, 1910

Winternitz: See abstract in Zent. Chir., 35» 314» 1903

Samef as above. Pyonephrosis of opposite hal .

m

Nephrotomy for calculus.

/ Nephrotomy for multiple calculi.

Zondek: Deut. Med. Woch., Oct. 13, 1921, See orig. again

Pyelotomy for calculus. Diagnosis of horseshoe kidney made before operation by presence of shadows of both kidneys close to spine.

29

Voorhoeve: Jour. de Radiol., 3, 414, 1919

Pyelotomy for calculi. Diagnosis before operation from facts that both kidney shadows were close to spine, were verti ' cal and both lower (at same level) than normal.

30 Rathbunzhjour. Urol., 12,612, Dec., 1924

i

Pyelotomy for calculus. Post-operative pyelogram confirmed diagnosis horseshoe kidney made at time of operation.

genstr., 29, 808, 1922

31 Carlierz Memoires d’Urologie, July, Resection of tuberculosis upper third of 191 1, Masson & Co., Paris one-half. Recovery.

32 Lange: ANNALS OF SURGERY, 35, 581, Nephrotomy for calculi. Recovery. I901

33 Vince: Cercle Med., Brussels, 1902 Nephrotomy for calculus.

34 Walton: Ann. Genito-urin., 1802, 1910 Nephrotomy for calculus. Recovery.

35 Legueu: Traite Chirurg. d’Urol., 749, Pyelotomy for calculus. Recovery. 1910 H

.36 Reynard: Lyon Med., 132, 151, 1923 Nephrotomy for calculus.

37 Kraft: Fortsch. a.d. Geb. d. Roent- Nephrotomy for calculus. Possibility of

horseshoe kidney considered before operation because of proximity of shadow to spine.

TABLE IV Primary Pyelotomy or Nephrotomy and Secondary Heminephrectomy

fig Author and reference Operations and remarks

I Gerard: Ann. mal. gen. urin., 29, 684, Pyelotomy for multiple calculi. Secondary

Apr., I9II heminephrectomy for pyelonephritis. Died p.o.

2 Kuster: Cited by Kobylinski, Folia Pyelotomy for hydronephrosis. Secondary Urolog., 6, I29, I91 I heminephrectomy.

3 Israel: Fol. Urol., I, 617, 1908 Diagnosis before operation by palpation.

Nephrotomy for intermittent hydronephrosis. Secondary nephrectomy.

4 Idem. I:IephrotomyIfor hydronephrosis. 5 H Albarran: See Kobylinski Same as above. I 6 Socin: Beitr. Klin. Chir., 4, 197, I888 Nephrotomy for hydronephrosis. Second ary heminephrectomy.

7 Boeckel: Jour. d’Urol., 12,296, 1921 Pyelotomy for calculi, Secondary heminephrectomy for fistula due to ureteral calculus. ‘

8 Socin: (See Case 3 Table III) Primary nephrotomy for hydronephrosis. Secondary heminephrectomy. Death from hemorrhage.

9 Czerny-Nehrkom Beitr. Klin. Chir., 31, Nephrotomy for hydronephrosis. Secondary 139, 1900 nephrectomy.

IO Winternitz: See Steiner, Zent. Chir., Bilat. Nephrolithiasis of horseshoe kidney. 28, 314, I910 Nephrotomy for calculus followed by herninephrectomy on one side, nephrolithotomy on opposite side. Recovery.

TABLE V Plastics or Ureterolysis on Horseshoe Kidneys

Author and reference Operation and remarks

Case ' N o.

I G:egoire:3our. d’Urol., 1, 659, I914 I\/Iobilized kinked ureter causinghydronephrosis of half of horseshoe kidney. Recovery.

2 Judd, Braasch and Scholl, (loc. cit.) Division of isthmus with mobilization of ureter and rotation of right half in case of congenital hydronephrosis of one-half of horseshoe kidney.

wards, as it approached the shadow, but did not come in close contact with it (B of fig. 14). The left opaque catheter followed a similar course, but turned inwards at a level corresponding to that of the right-sided shadow. The right pyelogram? (C of fig. 14) revealed an elongated vertical pelvis close tolthe spine with an inferior calyx directed mesially, overlapping the disc between the third and fourth lumbar vertebrae. The left pyelogram was more laterally located, but also had a mesially directed calyx.

_ ’rWe are indebted to Doctor M_ahone, the resident genito-urinary surgeon for the painstaking manner in which the pyelograms were made in all of the cases. I

43 ‘ i753 Case . No.

TABLE VI Injuries of Horseshoe Kidneys

Author and reference

Case No.

Description and remarks

1 Ehier: \-Ni-en. K1. W., 59, 3211,-fiebi 6,

Z-4-;

Crushing injury of abdomen. T emponnade.

I909 Autopsy revealed tear of isthmus. 2 Brunner: Beitr. Klin. Chir., I22, I46, Heminephrectomy for rupture of one-half I92 I of horseshoe kidney.

3 Hinterstoisser: Wien. Klin. Woch., 33, 942, Oct., I920

-4 L. Herman: ].A.M.'A.-, ii3, IT],’I9;4, pp. 1315-1321 ‘

Crushing injury. Heminephrectomy. Death.

4:1 —:—n — —n

5 S. C. Dean: ANNALS or SURGERY, 75, 253,1922

Gunshot wound of hilus of right half. Heminephrectomy. Recovery.

TABLE VII Miscellaneous Cases

Author and reference

Lesion and remarks

I Moynihan: Brit. Med. ]our., 1, 263, Feb. I, 1902

Aspirated and removed wall of cyst of isthmus.

2 Pichler: Mitt. a. d. Grenz., geb., 30, 557,

Made diagnosis horseshoe kidney by pal 19I8 pation and confirmed at autopsy.

3* Idem. T Same.

4D Idem. Same.

5 éergteri 1\/lt. Sinai Hosp. Rep., 1, 214, Decapsulation for acute nephritis. Recovery. I 99 .

6 Ktittnerz Berl. Klin. Woch., 30, 471, Exploratory ‘ for chronic hemorrhagic I911 nephritis. Diagnosis before operation by

palpation.

7 Sturfndorfz llev. de Gyn. -et Chir. abd., 3. 1053» 1903

Mobile horseshoe kidney. Nephropexy.

i

8 Buss: Zeit. Elin. Med., 5:49, 189;)

N ephrectomy (through error) of entire horseshoe kidney lying in true pelvis.

1 u—’ Both ureters entered the respective pelves shadows of the mesially directed calyces. horseshoe kidney was made and confirmed the usual lumbar kidney incision forwards

in a peculiar manner, passing behind the From the above findings a diagnosis of at operation. It was necessary to extend so that the anterior surface of the renal

pelvis could be exposed after displacement inwards of the peritoneum. The upper pole was at the level of the costal arch and one could follow an isthmus of about 4 cm.

width inwards until it crossed the spine.

Through an incision in the anterior aspect

of the renal pelvis, much phosphatic detritus and two well-formed but soft calculi were

removed. No attempt was made to close the pyelotomy incision.

was uneventful.

The convalescence

CASE II.—P_\'t’]()f()IlI_\’ for renal califiilzis in 0izu—/zalf of Izorscslzuc /cicihzey. 1-’resence of I/zis czizoiizizly di'a_c/nosi'd before 0[m'atz'0n.

Male, aged fifty-oiie, with history of fistula following drainage of right perinephric abscess ten months hefore. There was marked 1); uria and absence of dye excretion from this right kidney, but clear urine and prompt concentrated (lye output on the left side. l\’a(liogi‘apliy (Dr. Cora M. Nlattliews) revealed a series of four oval shadows (‘A of fig‘. 15) on the left side and close to the spine. They were directed dowiiwards and iiiwards. so that the lowermost one was in Contact with the outer end of the left traitsVerse process of the fourth lumbar \'ertehra. The intrarenal character of these shadows was confirmed hy the relatioii of the opaque catheter and hy p_\'elog'rapli_\' (B of liig. 15‘). The former curved sharply iiiwarcls and at its upper end was in close contact with the lowermost of the calculous shadows. The opaque medium included all of the. shadows and revealed a narrow Vertical pelvis. with the upper calyx directed inesially. Froiii these tii1(liiigs alone a diagnosis of calculi in the left half of a horseshoe kidney was made. In order. however. to more accurately ascertain the condition of the right half. a p_\'elogram was made and revealed (C of fig. 15) an arlvanced degree of dilatation of the renal pelvis. thus contiriiiiiig our fiiiclings on ureteral catheterization. Before a right lieniinephrec— toniy could he considered it

was deemed a(lVlSal)le to H3" fiG. 13, A and B.——Most frequent types of blood-vessels (Papin). move the calculi from the A. Single vessel to each_ha1f and two to isthmus. B. Single vessel 1 ft 1 If to each half and one to isthmus.

L“ ' 18. .

On June 17, 1925, the left renal pelvis was exposed extraperitoneally, on its anterior aspect. The upper pole of this half of the horseshoe kidney was, as in the first case. at the level of the costal arch and the lower pole was continuous with an isthmus which measured 3 to 4 cm. in a vertical direction. The ureter, as in the first case, passed across the front of the isthmus and like the pelvis, showed marked thickening of its walls. No difficulty was experienced in the delivery of four calculi through an incision in the alltcrigr aspect of the renal pelvis. The convalescence from this operation was unevent 755 EISENDRATH, PHIFER AND CULVER

ful and an attempt will be made in the near future to remove the pyonephrotic right half. (C of fig. 15.)

CASE III.—Tuberculosis of one-half of a_ horseshoe kidney. Presence of this anomaly diagnosed _by pyelography but not yet confirmed at operation. Male, aged twenty-four. Sudden onset of severe pain over right kidney region of one

C week’s duration. Frequency of urination especially during the day for a longer period. There was considerable tenderness over the right kidney. One brother had kidney removed for tuberculosis. Bladder urine very turbid, as was also that from the left kidney. Dye excretion from this side was delayed and poor as compared with the opposite (right) side. Acid-fast bacilli were found by Doctor Connell, the interne in charge, in the bladder urine, but they could not be found in the urine from the left kidney. Radiography (Dr. Cora M. Matthews) revealed nothing D abnormal in the plain film, -i.e., before the opaque catheters were passed. The film taken after these (opaque catheters) were introduced and the opaque median (12 per cent. sodium iodid) injected on both sides revealed the following very interesting findings. (fig. 16.) I. The opaque catheters on both sides curve outwards as they reach the lower border of the fourth lumbar vertebra. This is more marked on the left side.

2. The right pyelogram has an unusual contour. At

its upper end one observes fiG. I3,Cand D.—Most frequenttypesof blood-vessels(Papin). Superior and middle calyces

eCa.C}T;lvao1fy::?l:rf§fgcglhglisand twotoisthmusl. D. Two vessels to which are app,-0Xjm.ate]y nor _mal in location but unusual in arising from an expanded area of the pelvis instead of a tapering portion as is to be seen in the normal pelvis. There is a rudimentary infer_ior calyx directed laterally. The most striking feature, however, of this right pyelogram is seen at its lower end. Here one notes the extension mesially of the pelvis proper, so that it completely covers the corresponding transverse process of the third lumbar vertebra. This portion of the pelvis is almost quadrilateral in form and has rudimentary calyces along its mesial and

fiG. 14. Radiographic and yelographic findings in Case I. A print—Shadow of r_ight renal calculus over outer end of right transverse process of second lumbar vertebra. B pr1nt—Note ow r1ght_ opaque catheter turns outward and left one mwards. C pr1nt—Note meslally d1rected calyces (see text) and unusual forms of both pyelograms; also close proximlty of fight one to spme. T

fiG. I5.—Radipgraphic'and pyelographjc findirggs in Case II. _ _ B. Pyelogram mcluding shadows _seen 1n A, Wlth several calyces d1rected mesxally. marked d1Iatat1on of pelvis and calyces (mfected hydronephrosis).

A. Shadows of the four calculi arranged in serial rr_1anner_obliquely opposite fourth lumbar verteNote pecullar shape of th1s pelv1s. C. Pyelogram of right half showing caudal borders. A diagnosis of horseshoe kidney could be made from such a pyelogram alone. __ . . _

3. The right ureter runs behind the inferior calyx and enters the pelvis along the middle of its curving caudal (inferior) border. .

4. The left pyelogram also reveals some features which are characteristic of horse R

fiG. I6.—Pye1ographic findings in Case III.’ Note mesially directed calyces on both sides; also howfright pelvis extends across front‘ of body of third lumbar vertebra. Note unusual form of both pelves. shoe kidney, due to faulty rotation. The pyelogram is situated at about the. distance from the spine which is found under normal conditions. The pelvis itself has a peculiar form, there being a marked protrusion along the mesial border at the upper inner angle. The superior middle and inferior major calyces are very short and the ureter as on the right side runs behind the inferior major calyx to enter the pelvis along the middle of its caudal (inferior) border, instead of its mesial as in the normal kidney.

A more significant finding is that one of the calyces is directed mesially, an almost pathognomonic evidence of renal torsion, as Braasch has pointed out.

From our pyelographic evidence we feel confident that we are dealing with a tuberculosis of one-half of a horseshoe kidney, but the patient having thus far refused operation, we must postpone confirmation of our diagnosis for the present.

RESUME OF ALL PUBLISHED CASES AND OUR OWN

I. Clinical Pictu-res.—Aside from the syndrome first described by Rovsing 1‘ there are no pathognomonic symptoms indicative of this anomaly. I 7In the cases first reported by ‘Rovsing and since by others (see Table I), the abdominal pains are thought to be due to pressure of the isthmus on the large vessels behind it (aorta and ‘vena cava) and accompanying them. The complete relief of symptoms after division of the isthmus (_symphysiotomy) lends support to this compression theory. The pain in these cases is referred to both lumbar regions and is vaguely localized in different parts of the abdomen. The most characteristic feature is the increased degree of pain on leaning forwards or upon exertion, and its complete disappearance on lying down. Neufville 15 described an unusual case related to this syndrome of Rovsing. A young man of twenty-five had

fiG. I7.—Hyd_ronephrosis of right half of horseshoe oedema Of l)OlZh IOWCI‘ and

kidney. (Bockenhe1mer.) . .

a slight degree of ascites for a brief period. At autopsy the vena cava was found thrombosed by the compression of a vena cava. We quote this case with skepticism as to the relation of the anomaly to the thrombosis.

In Table IX we have grouped the entire I 32 cases, including our own, as to the frequency of the various lesions and would direct attention to the fact that the majority‘ are,‘ the result of the conditions mentioned above as being present in horseshoe "kidney and favoring stagnation. For this reason, diseases such as calculi, l1yd1'o- and pyonephrosis, etc., constitute the majority.

2. Diagnosis.—(Compare with Table VIII.)

In the earlier cases, the

proportions which were diagnosed by palpation alone is far greater than since the advent of radiography supplemented by pyelography. Of a total of I 33 cases) (including our first two) only 19, or 14.2 per cent., were diagnosed before operation or autopsy and confirmed. From the modern urologic‘ standpoint we can eliminate the ten cases (all except Van Houten in the first

TABLE VIII Cases Diagnosed before Operation or Autopsy?

By proximity

Proximity

Palpation or plus ?e¥1aI1’1;%’;iCrlr:)i:"v3; calculus BY calculus Suspected pyelography befo e pyelography to spine fiiaggigg alone stlgaggigg operatlion Martinow, I—-2. . . . . Zondek, III—28 — Tudd, Braiasch Papin, I:I I -Ju-dd. éfiaasai Kr-ogs, I:9- an an Rovsing, I-3 . . . . . . . Voorhoeve, III—I9 Scholl, III—I9 Scholl, III—2I Rovsing. I-I3 Malmovsky, I-4. . . . . Rathbun, II—39 Van Houten (3) Eisendrath, Steiner, III—2 I-1 I . . . . . . . . . . . . Phifer and Culver. III—24 Israel, IV—3 . . . . . . . . idem, III—25 Israel, III—4r Pichler, (2). VII-2. . _ Pichler, (2). VII-3 . . Pichler, (2). VII—4. . Newman, III—I6. . .. Israel, II-69 . . . . . . . . Kuttner, II—6. . . . . Totals . . . . . . . . . .11 2 I 2 3 4 I figures after author's name refer to Table and Case number respectively. 2. Pichler’s cases were not operated, but confirmed at autopsy. 3. In this case diagnosis made by palpation and confirmed by pyelography. TABLE IX Frequency of Various Lesions Tlffbl Pain Hydronephrosis Calculi Tuberculosis Pyonephrosis Others 00

I I I ~ I

2 23 13 I3 6 ' 9

3 I 34 I

4 7 3

5 2

6 i 4

7 I 3 Totals. . . .. II 34 51 13 7 16

column of Table VIII) in which diagnosis was made by palpation alone because this would hardly be depended upon at the present time.

One can also discard the four cases not included in the nineteen (Table VIII) in which the diagnosis was only suspected, thus leaving nine cases in which more recent methods of diagnosisj: were employed. From an analysis of these nine cases we can cite the following as important radiographic features.

-2}: Radiography (plain) supplemented by employment of opaque catheter and pyelography.

(a) The close proximity of one or both renal shadows to the spine at a lower level than normal.

(b) The close proximity to (fig. I4) or obliquity of position (fig. I 5) in relation to the spine, of the shadows of renal calculi. If one or both halves of the horseshoe kidney lie close to the spine the value of (a) and (b) as diag fiG. I8.—Hydronephrosis of right half of horseshoe kidney with superior isthmus. (Karewski.)

nostic features cannot be underestimated. If, however, one or both halves are symmetric (fig. I), i.e., at the same level and as far away from the spine as is the normal kidney, the above data are of little value alone. One must also recall the possibility of renal or calculous shadows being at different levels (fig. 2) in an asymmetric horseshoe kidney.

(c) Urography.—This in our opinion is the method which corroborates the suspicions raised by the findings cited under (a) and (b). 7 If one or both pyelograms (figs. I4, 15 and I6) lie in close proximity to the spine at the same or different levels, or even extend partly across the spine (fig. 6), as in one of our own and in Rathbun’s case, there can be little doubt as to the presence of horseshoe kidney. The same is true even if one pyelogram is close to the spine and the opposite one at the normal distance. (fig. I4.) If, however, both pyelograms are not close to the spine one must depend on

other findings which are of great value not only under these conditions of normal distance of pyelo grams from the spine, but also when one or both are in close proximity.

These additional data were first called to our attention by Braasch. They are due to the faulty rotation of the halves of a horseshoe kidney. As a result we find (a) one or more calyces directed mesially (fig. I4); (b) very long, narrow pelves (fig. I5) or “ bizarre” shapes; (c) unusual course of the ureter, i.e., passing behind a calyx (fig. 14) and not entering the pelvis along its convex border. (fig. I4.)

‘Ne believe that nu-‘re fiG. I9.—Hydronephrosis of both halves of ahorseshoe kidney. . P ' . widespread knowledge of s ( "“p“’) . these radiographic features will enable us-to make a pre—operative diagnosis

in the future in a larger percentage of cases.

TYPES or OPERATIONS PERFORMED“

Table I. Symphysiotomy (Division of isthmus), alone or combined with other operations, such as fixation of left half after pyelotomy

for calculi (Egger’s case) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. I2 cases Table II. Heminephrectomy alone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 63 cases Table III. Pyelotomy or nephrotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 35 cases Table IV. Primary pyelotomy or nephrotomy and secondary heminephrcctomy 10 cases Table V. Plastics or ureterolysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 2 cases Table VI. Subparietal injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 4 cases

Table VII. Miscellaneous, not including three cases of Pichler (not operated) . 5 cases

Total . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. I3I cases

1] Owing to omission of mention of result of operation in_a su_fficient.ly_ large number to nullify the value of any deductions, the percentages of deaths and recoveries Wlll be omitted.

Technic of Operations on Horseshoe Kidneys.--The method of approach should always be by the extraperitoneal route employing the same incision (lumbar) as in the normally placed and formed kidney. It is necessary, how fiG. 2o.—Bilateral calculi in horseshoe kidney. (Schuchardt.)

ever, to extend the incision much nearer the outer border of the corresponding rectus muscle because access to the pelvis must be from the ventral and not from the dorsal aspect as in ordinary (pos terior) pyelotomy. There is usually no difficulty in displacing the peritoneum while the patient is in the lateral position and then changing to a supine position while the pelvis and isthmus are being exposed. We found that this change of position of the patient after division of the various layers of the abdominal wall and strong retraction of the peritoneum enabled us to work under guidance of the eye in both cases. For heminephrec— tomy a similar good exposure is essential owing to the many accessory vessels (both arteries and veins) which enter the hilus, poles and isthmus in a very irregular manner. The isthmus can be clamped as one proceeds to divide it and the denuded areas closed by mattress sutures of chromic gut reinforced at loop and knot by fat pads.

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